Malignus kevert Müller-cső-eredetű tumor komplex kezelése = Multimodal treatment of malignant mixed Müllerian tumor

Absztrakt: Bevezetes es celkitűzes: A kozlemeny celja ismertetni a mehdaganatok egy meglehetősen ritka, klinikailag igen agressziv tipusanak, a malignus kevert Muller-cső-eredetű daganatnak, mas neven carcinosarcomanak a jellegzetes prognosztikai faktorait, kezelesenek lehetősegeit. Modszer: 2009 es 2017 kozott 29 beteget kezeltunk malignus Muller-cső-eredetű tumor miatt. I. stadium eseten műtetet es posztoperativ sugarkezelest vegeztunk. II–IV. stadium eseten trimodalis kezeles tortent (műtet, kemoterapia es sugarkezeles). Eredmenyek: A betegek atlageletkora 68,51 (49–90) ev, atlagos BMI: 30,22 (20,90–37,22). Komplett reszekcio utani recidivat 6 esetben diagnosztizaltunk (ebből 4 beteg nem fogadta el a sugarkezelest), atlagosan 15,52 (6–36) honap elteltevel, tavoli metasztazist 5 esetben, atlagosan 19,2 (8–32) honap mulva. A teljes tuleles atlag 11,92 honap (1–75). Hat beteg jelenleg is daganatmentes. Kovetkeztetesek: Jelenleg nincs egyseges konszenzus a daganat terapias ellatasara vonatkozoan. A kezelesben standard a műteti eljaras, mely teljes hasi meheltavolitast es ketoldali adnexectomiat jelent, azonban a helyi recidivak es tavoli metasztazisok nagy előfordulasi aranya miatt felmerult a regionalis nyirokcsomo-eltavolitas es posztoperativ kezeles szuksegessege. Bar a posztoperativ sugarkezeles a lokoregionalis kontrollt javitja, tulelesre vonatkoztatott előnye tovabbra sem bizonyitott. Az adjuvans kemoterapia mind a kismedencei, mind az extrapelvicus recidivak aranyat csokkenti, azonban tovabbra sincs egyertelmű ajanlas a leghatekonyabb kemoterapias szerre vonatkozoan. A teljes tulelest kombinalt citosztatikus kezelessel sem sikerult javitani az elmult evtizedekben, ezert azt gondoljuk, hogy a multimodalis kezelestől varhatok jobb eredmenyek. A hatekonyabb ellatas celjabol – az onkologia mas teruleteihez hasonloan – biologiai terapiaval es target kezelessel kapcsolatban is folynak vizsgalatok; az alacsony betegszam miatt relevans kovetkeztetes csak hosszu evek mulva vonhato le. Orv Hetil. 2018; 159(19): 741–747. | Abstract: Introduction and aim: The aim of our study was to evaluate the prognostic factors and treatment options of a very rare and highly aggressive type of uterine neoplasms, the malignant mixed Mullerian tumor, known as carcinosarcoma. Method: Between 2009 and 2017, 29 patients were treated with malignant mixed Mullerian tumor. At stage I, surgery and postoperative radiotherapy were performed. At stages II−IV, trimodal treatment (surgery, chemotherapy and radiotherapy) was administered. Results: The average age of patients was 68.51 (49–90) years, mean body mass index was 30.22 (20.90–37.22). We have experienced recurrence of disease after complete resection in 6 cases (4 of 6 patients did not accept radiation therapy). Local recurrence has occurred after an average 15.52 (6−36) months, distant metastasis with an average 19.2 (8–32) months. Overall survival was 11.92 (1–75) months. Six patients are free of tumours at the moment. Conclusions: As overall survival has not increased in recent decades by using combined chemotherapy, there is no congruent consensus associated with the optimal treatment. The standard surgical treatment is total abdominal hysterectomy with bilateral oophorectomy, although due to high rates of recurrence and metastases, the necessity of lymphadenectomy and postoperative treatment is in the focus of recent studies. Though postoperative irradiation improves local control, the beneficial effect on overall survival is still not proven. Adjuvant chemotherapy decreases the rate of both pelvic and extrapelvic recurrence at the same time, although there is no recommendation for the optimal chemoterapeutic agent. Multimodal therapy should lead to better outcomes. Recently there are many ongoing studies with biologic and target therapies to improve efficiency, however, the relevant results will be disclosed in many years only, due to the small number of patients. Orv Hetil. 2018; 159(19): 741–7747.